Summary
We examined the oculomotor and/or trochlear nuclei of 27 amytrophic lateral sclerosis (ALS) patients and 10 controls by histological and immunohistological methods. Their neurons were relatively well preserved. In 7 of 22 sporadic ALS patients (including 3/3 ALS with ophthalmoplegia) and in 4 of 5 ALS patients with dementia, some morphological changes similar to those in anterior horns (Bunina bodies, ubiquitin-positive skein-like inclusions, Lewy body-like inclusions, conglomerate inclusions and spheroids) were rarely, but clearly seen. These changes were not observed in controls. Our results suggest that the oculomotor and trochlear nuclei in ALS patients are slightly affected in a manner similar to that in the anterior horns, but the degree is less than that necessary for development of ophthalmoplegia in the majority of ALS patients.
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References
Carpenter S (1986) Proximal axonal enlargement in motor neuron disease. Neurology 18:841–851
Esteban A, DeAndres C, Gimenetz-Roldan S (1978) Abnormalites of Bell's phenomenon in amyotrophic lateral sclerosis. A clinical and electrophysiological evaluation. J Neurol Neurosurg Psychiatry 41:690–698
Feigin I (1991) Astrocytic changes in white matter of ALS brain. J Neuropathol Exp Neurol 50:678–679
Harvey DG, Torack RM, Rosenbaum HE (1979) Amyotrophic lateral sclerosis with ophthalmoplegia. Arch Neurol 36:615–617
Hayashi H, Kato S (1989) Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. J Neurol Sci 93:19–35
Hirano A, Inoue E (1979) Early pathological changes of amyotrophic lateral slcerosis. Electron microscopic study of chromatolysis, spheroids and Bunina bodies. Neurol Med (Tokyo) 13:148–160
Iwata M, Mannen T, Inoue K (1991) Functional anatomy of the Onufrowicz nucleus in man. In: Mannen T (ed) Annual report of the Research Committee of CNS Degenerative Disease. The Ministry of Health and Wellfare of Japan, Tokyo, pp 207–210
Jacobs L, Bozian D, Heffner RR Jr, Barron SA (1981) An eye movement disorder in amyotrophic lateral sclerosis. Neurology 31:1282–1287
Kurshner MJ, Parrish M, Burke A, Behrens M, Hays AP, Farme B, Rowland LP (1984) Nystagmus in motor neuron disease: clinicopathological study of two cases. Ann Neurol 16:71–77
Kusaka H, Matsumoto S, Imai T (1990) An adult-onset case of sporadic motor neuron disease with basophilic inclusions. Acta Neuropathol80:660–665
Kushner PD, Stephenson DT, Wright S, (1991) Reactive astrogliosis in widespread in the subcortical white matter of amyotrophic lateral brain. J Neuropathol Exp Neurol 50:263–277
Lawyer T Jr, Netsky MG (1953) Amyotrophic lateral sclerosis. A clinicoanatomic study of fifty-three cases. Arch Neurol Psychiatry 69:171–192
Leveille A, Kiernan J, Goodwin JA, Antel J (1982) Eye movements in amyotrophic lateral sclerosis. Arch Neurol 39:684–686
Mann DMA, Yates PO (1974) Motor neurone disease: the nature of the pathogenic mechanism. J Neurol Neurosurg Psychiatry 37:1036–1046
Mannen T, Iwata M, Toyokura Y, Nagashima K (1977) Preservation of a certain motoneuron group of the sacral cord in amyotrophic lateral sclerosis: its clinical significance. J Neurol Neurosurg Psychiatry 40:464–469
Mizutani T, Aki M, Shiozawa R, Unakami M, Nozawa T, Yajima K, Tanabe H, Hara M (1990) Development of ophthalmoplegia in amyotrophic lateral sclerosis during long-term use of respirators. J neurol Sci 99:311–319
Mizutani T, Sakamaki S, Tsuchiya N, Kamei S, Kohzu H, Horiuchi R, Ida M, Shiozawa R, Takasu T (1992) Amyotrophic lateral sclerosis with ophthalmoplegia and multisystem degeneration in patients on long-term use of respirators. Acta Neuropathol 84:372–377
Murayama S, Mori H, Ihara Y, Bouldin TW, Suzuki K, Tomonaga M (1990) Immunocytochemical and ultrastructural studies of lower motor neurons in amyotrophic lateral sclerosis. Ann Neurol 27:137–148
Oda M, Akagawa N, Tabuchi Y, Tanabe H (1978) A sporadic juvenile case of the amyotrophic lateral sclerosis with neuronal intracytoplasmic inclusions. Acta Neuropathol (Berl) 44:211–216
Okamoto K, Hirai S, Ishiguro K, Kawarabayashi T, Takatama M (1991) Light and electron microscopic and immunohistochemical observations of the Onuf's nucleus of amyotrophic lateral sclerosis. Acta Neuropathol 81:610–614
Okamoto K, Hirai S, Shoji M, Harigaya Y, Fukuda T (1991) Widely distributed Bunina bodies and spheroids in a case of atypical sporadic amyotrophic lateral sclerosis. Acta Neuropathol 81:349–353
Okamoto K, Murakami N, Kusaka H, Yoshida M, Hashizume Y, Nakazato Y, Matsubara E, Hirai S (1992) Ubiquitin-positive intraneuronal inclusions in the extra-motor cortices of presenile dementia patients with motor neuron disease. J Neurol 239:426–430
Takahata N, Yamanouchi N, Fukatsu R, Katagiri M (1976) Brain stem gliosis in a case with clinical manifestations of amyotrophic lateral sclerosis. Folia Psychiatr Neurol Jpn 30:41–48
Tomonaga M (1980) Selective appearance of Bunina bodies in amyotrophic lateral sclerosis. J Neurol 223:259–267
Van Bogaert L (1925) Contribution à la connaissance des troubles oculaires et vestibularies dans la sclérose latérale amyotrophique. Rev Oto-neurooculist 3:263–274
Yoshida M, Murakami N, Hashizume Y, Itoh E, Takahashi A (1992) A clinicopathological study of two respirator-aided long-survival cases of amyotrophic lateral sclerosis. Clin Neurol (Tokyo) 32:259–265
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Supported by The Nakabayashi Trust for ALS Research and by The Ministry of Health and Welfare of Japan
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Okamoto, K., Hirai, S., Amari, M. et al. Oculomotor nuclear pathology in amyotrophic lateral sclerosis. Acta Neuropathol 85, 458–462 (1993). https://doi.org/10.1007/BF00230482
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DOI: https://doi.org/10.1007/BF00230482